Splenic rupture as the presenting manifestation of vasculitis

Although underreported, histologic splenic involvement in Wegener's granulomatosis (WG) is not unusual. Splenic rupture in association with WG, however, is rare. Only 2 cases of nontraumatic splenic rupture have been reported as the initial feature of WG. Isolated cases of splenic rupture also have been noted in rheumatoid arthritis, systemic lupus erythematosus, and polyarteritis nodosa.

To report the third case of splenic rupture as the presenting feature of WG and review the literature concerning splenic rupture in other rheumatologic diseases to better delineate a mechanism for this rare occurrence.

Descriptive case report of 1 patient with WG with antecedent splenic rupture and a review of the relevant literature using a MEDLINE search from 1950 to 2001.

Our patient presented with symptoms and signs of WG 2 weeks after nontraumatic splenic rupture. Two similar cases have been reported: one showed splenic vasculitis histologically and the other only a neutrophilic infiltration at the site of the splenic tear and subcapsular zone after surgery. Although splenic capsular and pulp hemorrhage alone without signs of vasculitis were noted in our patient, no other cause (ie, hematologic, infectious, neoplastic, or otherwise) for splenic rupture was found.

As in the 2 reported cases, WG may have been responsible for splenic rupture in our patient. Regardless, early evaluation for connective tissue disease in a patient with spontaneous splenic rupture without apparent cause merits consideration, as it may affect patient follow-up and treatment.